To assess the prevalence and pattern of myocardial fibrosis as detected by delayed enhanced magnetic resonance imaging (DE-MRI) in patients with systemic sclerosis (SSc), and to evaluate a possible association between myocardial fibrosis and cardiac arrhythmias. Forty-one patients with SSc underwent 24-hour Holter monitoring, Doppler echocardiography, and DE-MRI following gadolinium administration. Technically acceptable DE-MRIs were obtained in 36 patients with SSc. Enhancement on DE-MRI, consistent with myocardial fibrosis, was observed in 24 of these patients (66%), and it was invariably midwall with a linear pattern, mostly involving basal and midcavity segments of the left ventricle. The volume of enhancement (total volume percentage index [TVPI]) did not differ between patients with diffuse SSc and those with limited SSc (mean +/- SD 1.46 +/- 1.73% versus 1.44 +/- 1.77%; P = 0.98). Patients with a long duration (> or = 15 years) of Raynaud's phenomenon had a greater number of enhancing segments (mean +/- SD 6.55 +/- 4.93 versus 2.96 +/- 3.46; P = 0.017) and a greater TVPI (mean +/- SD 2.44 +/- 1.97% versus 1.02 +/- 1.43%; P = 0.02) than those with a duration of Raynaud's phenomenon <15 years. Nineteen patients with SSc (53%) had abnormal Holter study results. Compared with patients with normal Holter study results, those with abnormal results had a greater number of enhancing segments (mean +/- SD 5.4 +/- 4.8 versus 2.5 +/- 2.9; P < 0.05) and a greater TVPI (mean +/- SD 2.1 +/- 1.9% versus 0.8 +/- 1.2%; P < 0.05). DE-MRI can identify myocardial fibrosis in a significant percentage of patients with SSc and may be a useful noninvasive tool for determining cardiac involvement.